The Clinical Course of Patients with Trisomy 18 Who Have Not Undergone Cardiac Surgery

• Imai Yuki

  Department of Pediatrics, Anjo Kosei Hospital

• Kato Taichi

  Department of Pediatrics, Nagoya University Hospital

• Kato Yuichi

  Department of Pediatrics, Anjo Kosei Hospital

• Kubota Tetsuo

  Department of Pediatrics, Anjo Kosei Hospital

• Hattori Tetsuo

  Department of Pediatrics, Anjo Kosei Hospital

Bibliographic Information

Other Title

• 先天性心疾患の手術非介入で経過している18トリソミーの検討

Abstract

Background: The number of patients with trisomy 18 undergoing cardiac surgery has recently been increasing. In our institute, the primary purpose of treatment in patients with trisomy 18 is to facilitate discharge and cardiac surgery has not yet been performed in such cases. In the present report, we reviewed patients treated at our institute to demonstrate the prognosis of patients with trisomy 18 without cardiac surgery. Methods: We collected detailed clinical information from the medical records of 17 patients with trisomy 18 from April 2005 to December 2015. We retrospectively studied the neonatal condition, survival rate, and cause of death. We also reviewed patients with a high pulmonary flow, the large left-to-right shunt (LS) group, who were at a potential risk of heart failure. Results: All patients had congenital heart disease (CHD). The 1-year survival rate was 64.7%, with 70.6% of patients discharged at a median age of 129 days (range, 16–285 days). The median survival time of patients who died during the present study was 104.5 days (range, 0–1,054 days). In the LS group, 62.5% of patients survived for one year with no evidence of heart failure. Two patients in the LS group died in hospital from heart failure. Conclusions: Favorable survival was achieved through standard neonatal intensive treatment without cardiac surgery. Patients with trisomy 18 complicated by CHD with a high pulmonary flow should be maintained in a stable condition without cardiac surgery due to persistent pulmonary hypertension resulting from vascular abnormalities. Further studies are required to evaluate the long-term effectiveness of cardiac surgery for patients with trisomy 18.

Journal

• Pediatric Cardiology and Cardiac Surgery

  Pediatric Cardiology and Cardiac Surgery 33 (4), 312-317, 2017

  Japanese Society of Pediatric Cardiology and Cardiac Surgery

Keywords

• trisomy 18
• congenital heart disease
• cardiac surgery
• survival rate

Details

• CRID: 1390001204670821504
• NII Article ID: 130006003937
• DOI: 10.9794/jspccs.33.312
• ISSN: 21872988
• ISSN: 09111794
• Web Site: https://www.jstage.jst.go.jp/article/jspccs/33/4/33_2017-0018/_pdf
• Web Site: https://search.jamas.or.jp/link/ui/2018003130
• Text Lang: ja
• Data Source:
  • JaLC
  • Crossref
  • CiNii Articles
  • KAKEN
• Abstract License Flag: Disallowed